Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members.

@article{Yawn2014ManagementOS,
  title={Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members.},
  author={Barbara P. Yawn and George R. Buchanan and Araba N Afenyi-Annan and Samir K. Ballas and Kathryn L. Hassell and Andra H. James and Lanetta B Jordan and Sophie M. Lanzkron and Richard Lottenberg and William J. Savage and Paula Tanabe and Russell E. Ware and M. Hassan Murad and Jonathan C. Goldsmith and Eduardo Ortiz and R Fulwood and Ann Horton and Joylene John-Sowah},
  journal={JAMA},
  year={2014},
  volume={312 10},
  pages={
          1033-48
        }
}
IMPORTANCE Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100,000 individuals in the United States and is associated with many acute and chronic complications requiring immediate medical attention. Two disease-modifying therapies, hydroxyurea and long-term blood transfusions, are available but underused. OBJECTIVE To support and expand the number of health professionals able and willing to provide care for persons with SCD. EVIDENCE REVIEW Databases of… Expand

Paper Mentions

Interventional Clinical Trial
To determine the feasibility of matching donor red cells by RH genotype for a cohort of chronically transfused patients with SCD.  
ConditionsSickle Cells Disease
InterventionBiological
Interventional Clinical Trial
This is a pilot study to evaluate the feasibility and safety of providing RH genotype matched D+ Red Blood Cells (RBCs) to chronically transfused patients with sickle cell disease (SCD… Expand
ConditionsAnti-D Antibodies, Sickle Cell Disease
InterventionBiological
Interventional Clinical Trial
multicentric interventional biomedical research phase II, prospective, non-randomized evaluating a haploidentical marrow transplants after reduced-intensity conditioning and… Expand
ConditionsSickle Cell Disease
InterventionBiological
Observational Clinical Trial
Quantitative and prognostic evaluation of dense red blood cells in sickle cell children: preliminary single center study from the Creteil pediatric cohort.  
ConditionsSickle Cell Disease
Evidence gaps in the management of sickle cell disease: A summary of needed research
TLDR
An NHLBI expert panel recently published evidence-based guidelines for the care of people with sickle cell disease, and identified many shortcomings in the available evidence that are summarized in this article and reflect the panel’s opinions, expertise, and experience in all aspects of care. Expand
Sickle Cell Disease: Prevention of Complications
TLDR
By presenting the following cases, the most important evidence-based guidelines and existing controversies in the prevention of SCD complications are reviewed, following the standard nomenclature of sickle cell anemia. Expand
Perioperative care of children with sickle cell disease: A systematic review and clinical recommendations
TLDR
A systematic PRISMA‐based review of the existing literature and ancillary practice is provided and a set of clinical recommendations and priorities for research are delineated. Expand
Implementation of the therapeutic use of hydroxyurea for sickle cell disease management in resource-constrained settings: a systematic review of adoption, cost and acceptability
TLDR
This review rigorously and systematically assessed the evidence on implementation of hydroxyurea in resource-constrained settings such as LMICs and found that knowledge regarding implementation is low. Expand
Evidence-based dental management in the new era of sickle cell disease: A scoping review.
TLDR
Systemic treatment of SCD might have opposing effects on caries, perhaps explaining the conflicting results published, and Penicillin, vaccines, and hydroxyurea might impact caries and bone. Expand
American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain.
TLDR
These evidence-based guidelines developed by the American Society of Hematology are intended to support patients, clinicians, and other health care professionals in pain management decisions for children and adults with SCD. Expand
American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease.
TLDR
These evidence-based guidelines of the American Society of Hematology are intended to support patients, clinicians, and other health care professionals in their decisions about screening, diagnosis, and management of cardiopulmonary and renal complications of SCD. Expand
Hydroxyurea can be used in children with sickle cell disease and cerebral vasculopathy for the prevention of chronic complications? A meta-analysis
TLDR
A systematic review for evaluating the impact of hydroxyurea and chronic blood transfusion in children with sickle cell disease found that the primary outcome was the occurrence of stroke and there is a lack of high-quality research in the care of children with SCD. Expand
Blood transfusions for treating acute chest syndrome in people with sickle cell disease.
TLDR
There is currently no reliable evidence to support or refute the perceived benefits of blood transfusions as treatment options; very limited information about any of the potential harms associated with these interventions or indeed guidance that can be used to aid clinical decision making. Expand
Blood transfusions for treating acute chest syndrome in people with sickle cell disease.
TLDR
There is currently no reliable evidence to support or refute the perceived benefits of blood transfusions as treatment options; very limited information about any of the potential harms associated with these interventions or indeed guidance that can be used to aid clinical decision making. Expand
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References

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TLDR
Although significant strides have been made in the care of children with SCD in the past 2 decades, more research needs to be performed, especially for acute events associated withSCD, to ensure that the health and well-being of childrenwith SCD continues to improve. Expand
Hydroxyurea for Sickle Cell Disease: A Systematic Review for Efficacy and Toxicity in Children
TLDR
Hydroxyurea reduces hospitalization and increases total and fetal hemoglobin levels in children with severe sickle cell anemia and there was inadequate evidence to assess the efficacy of hydroxyurea in other groups. Expand
How I use hydroxyurea to treat young patients with sickle cell anemia.
TLDR
Current evidence suggests that many young patients with SCA should receive hydroxyurea treatment, as recent evidence documents sustained long-term benefits with prevention or reversal of chronic organ damage. Expand
Systematic Review: Hydroxyurea for the Treatment of Adults with Sickle Cell Disease
TLDR
A systematic review of the available data on the efficacy, effectiveness, toxicity, and barriers to the use of hydroxyurea in people with sickle cell disease found that repeated transfusions of red blood cells can greatly decrease disease severity and hematopoietic stem cell transplantation can cure it. Expand
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TLDR
An overview of the process to establish consensus definitions of the most frequently occurring complications of Sickle Cell Disease is provided and use of these definitions will support greater accuracy in genotype–phenotype studies, thereby achieving a better understanding of SCD pathophysiology. Expand
Sickle cell disease: time for a closer look at treatment options?
TLDR
It is believed that it is time to broaden the use of definitive therapy for those with asymptomatic disease, being cautiously more aggressive in the approach. Expand
Morbidity associated with sickle cell disease in pregnancy.
TLDR
Women with sickle cell disease are at greater risk for morbidity in pregnancy than previously estimated. Expand
The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS).
TLDR
The results highlight the beneficial effect of HU, which seems to modify the natural history of SCD and raise the issue of expanding its use in all SCD patients. Expand
Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG)
TLDR
Hydroxycarbamide can now be considered for all very young children with sickle-cell anaemia, with some evidence for decreased acute chest syndrome, hospitalisation rates, and transfusion. Expand
Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment.
TLDR
Adult patients taking hydroxyurea for frequent painful sickle cell episodes appear to have reduced mortality after 9 of years follow-up, and indications for hydroxyUREa treatment should be expanded. Expand
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